Nigeria has a population of 160 million. 3% of them have sickle cell disease (SCD). Can you calculate how many people that is? That is 4.8 million people. Over 4 million individuals, representing by far the largest number globally. Most of these are children; children that do not survive to be adults, but could. They could survive if they had access to a cure as simple as one called bone marrow transplant (BMT).
Dr. Godwin Bazuaye, Head of the Stem Cell Transplantation Unit, at the Department of Hematology, University of Benin Teaching Hospital, in collaboration with the University of Basel-Switzerland, were the first pioneers to use bone marrow transplantation (BMT) for curing sickle cell anemia in Switzerland.
Cure2Children had the opportunity to spend some time with him during the Sickle Cell Disease (SCD) Consortium meeting in Regensburg, Germany last June.
Together, we plan to develop 6 initial bone marrow transplant centers in Nigeria that will primarily focus on SCD. We can’t wait!
Now, you might be wondering: “What exactly is sickle cell disease?
Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have a typical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.
Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.
The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with sickle cell disease and can lead to heart failure.
Facts about SCD:
- About 1 out of every 12 African-Americans has sickle cell trait and about 1 out of every 100 Latinos has sickle cell trait. Sickle cell trait also affects many people whose ancestors came from Africa, Latin America, Asia, India, and the Mediterranean region. However, it is possible for a person of any race or nationality to have sickle cell trait.
- Even though the WHO has recognized the size of the problems and it's increasing burden, very little has been done so far.
- Children born with sickle-cell anemia suffer from chronic pain episodes when the rigid sickle-shaped blood cells block small blood vessels. They also experience slow growth due to low iron levels, vision problems, enlarge sleep, stoke, and death. Treatment for sickle-cell disease is a life long battle. A one-time Bone Marrow Transplantation is the only existing cure; after which the child will go on to live an entirely normal and healthy life.